l-Carnitine is Synthesized in the Human Fetal–Placental Unit: Potential Roles in Placental and Fetal Metabolism

Oey, Nadia A.; Vlies, Naomi van; Wijburg, Frits A.; Wanders, Ronald J. A.; Attié‐Bitach, Tania; Vaz, Frédéric M.

doi:10.1016/j.placenta.2005.10.002

Cited by 45 publications

(35 citation statements)

References 46 publications

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“…Alternatively, fronto-temporal degeneration and chronic subdural effusion in GA I might be the effect of 3-OHGA on endothelial structures during brain development with subsequent vascular dysfunction [14]. Fetal maturation could also be influenced by the low levels of maternal carnitine [2,15,16], although it seems that the fetus is capable of independent carnitine synthesis in utero [17]. Only long-term follow up will permit us to determine the true impact of GA I during pregnancy.…”

Section: Discussionmentioning

confidence: 99%

Outcome of three cases of untreated maternal glutaric aciduria type I

Garcia¹,

Martins

Diogo³

et al. 2007

Eur J Pediatr

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We report, for the first time, the outcome of three children born to two women with untreated glutaric aciduria type I (GA I). Isolated hypocarnitinemia in neonatal screening in one baby allowed the identification of the disease in his mother, who was undiagnosed so far and had had a previous daughter. The other baby was born to an already diagnosed mother who was not treated; newborn screening in the child reflected the metabolic state of the mother. Biochemical abnormalities returned to normal within one week. At the age of 4 months, neuroimaging showed Sylvian enlargement in both infants and bilateral temporal arachnoid cysts in one. Physical and neurological developments were normal for the three patients at ages 2 and 5 years. We conclude that long-term follow up will determine the true impact of GA I in such children.

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Section: Discussionmentioning

confidence: 99%

Outcome of three cases of untreated maternal glutaric aciduria type I

Garcia¹,

Martins

Diogo³

et al. 2007

Eur J Pediatr

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“…Although direct evidence for the effect of carnitine supplementation is lacking, some beneficial effects on physical fatigue and exercise recovery have been reported (24,49,50). Fatty acid oxidation, and therefore also the availability of carnitine, has been shown to play an important role during fetal development [discussed by (51)]. OCTN2 is present in the human placenta and mediates fetal uptake of carnitine, but both the human fetal liver, kidney, spinal cord and placenta where also shown to synthesize carnitine (51).…”

Section: Carnitine Acquisition: Intake Versus Biosynthesismentioning

confidence: 99%

“…Fatty acid oxidation, and therefore also the availability of carnitine, has been shown to play an important role during fetal development [discussed by (51)]. OCTN2 is present in the human placenta and mediates fetal uptake of carnitine, but both the human fetal liver, kidney, spinal cord and placenta where also shown to synthesize carnitine (51). However, the availability of TML might be limiting as carnitine supplements have been shown to increase fetal growth in pigs (52,53) and human maternal plasma carnitine levels decrease during early pregnancy (54).…”

Section: Carnitine Acquisition: Intake Versus Biosynthesismentioning

confidence: 99%

Enzymology of the carnitine biosynthesis pathway

2010

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SummaryThe water-soluble zwitterion carnitine is an essential metabolite in eukaryotes required for fatty acid oxidation as it functions as a carrier during transfer of activated acyl and acetyl groups across intracellular membranes. Most eukaryotes are able to synthesize carnitine endogenously, besides their capacity to take up carnitine from the diet or extracellular medium through plasma membrane transporters. This review discusses the current knowledge on carnitine homeostasis with special emphasis on the enzymology of the four steps of the carnitine biosynthesis pathway.

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“…Developmental changes in the expression and activity of fatty acid oxidation enzymes at various gestational ages have been demonstrated recently in the human placenta and fetus (52-56). Oey et al could show for the first time that placental and fetal tissues are capable of complete carnitine biosynthesis and that the fetus is able to synthesize carnitine already from the 8th week of development (61).…”

Section: Energy Deficiency Typementioning

confidence: 99%

Impact of selected inborn errors of metabolism on prenatal and neonatal development

Illsinger

2010

IUBMB Life

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SummaryIn general, data regarding maturational processes of different metabolic pathways in the very vulnerable fetal and neonatal period are rare. This review is to substantiate the impact of selected inborn errors of metabolism on this critical period of life and their clinical manifestation. Significant adaptation of mitochondrial/energy-, carbohydrate-, lysosomal-, and amino acid-metabolism occurs during early prenatal and neonatal development. In utero, metabolic environment has an impact on the development of the fetus as well as fetal organ maturation.

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l-Carnitine is Synthesized in the Human Fetal–Placental Unit: Potential Roles in Placental and Fetal Metabolism

Cited by 45 publications

References 46 publications

Outcome of three cases of untreated maternal glutaric aciduria type I

Outcome of three cases of untreated maternal glutaric aciduria type I

Enzymology of the carnitine biosynthesis pathway

Impact of selected inborn errors of metabolism on prenatal and neonatal development

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